Bibliography

Bastian, F. O. (2005). Spiroplasma as a candidate agent for the transmissible spongiform encephalopathies. J Neuropath Exp Neurol 64, 833-838.

Dickson, D. (Ed.). (2003). Neurodegeneration: the molecular pathology of dementia and movement disorders (pp. 282-338). Basel: ISN Neuropath Press.

Epstein, L. G., & Brown, P. (1997). Bovine spongiform encephalopathy and a new variant of Creutzfeldt-Jakob disease. Neurology 48, 569-571.

Glatzel, M., Stoeck, K., Seeger, H., et al. (2005). Human prion diseases. Arch Neurol 62, 545-552.

Kovacs, T., Aranyi, Z., Szirmai, I., & Lantos, P. L. (2002). CreutzfeldtJakob disease with amyotrophy and demyelinating polyneuropathy. Arch Neurol 59, 1811-1814.

Kropp, S., Shultz-Schaeffer, W. Y., Finkenstaedt, M., et al. (1999). The Heidenhain variant of Creutzfeldt-Jakob disease. Arch Neurol 56, 55-61.

Prusiner, S. B. (1993). Genetic and infectious prion diseases. Arch Neurol 50, 1129-1153.

Schonberger, L. B. (1998). New variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy. Infect Dis Clin North Am 12, 111-121.

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- REVIEW QU

1. The infectious prion protein (PrPsc) is characterized by the following statement(s):

A. It consists of protein and nucleic acid.

B. It is a pathogenic isoform of a nonpathogenic prion protein (PrPc).

C. It evokes an inflammatory reaction with lymphocytes.

E. None of these.

2. The prion protein gene (PRNP):

A. Is located on chromosome 20

B. Is located on chromosome 26

C. At codon 130, it codes for methionine

D. At codon 129, it codes for either methionine or valine

E. None of these

3. Diagnostic abnormalities encountered in Creutzfeldt-Jakob disease are:

A. Vacuolated lymphocytes in circulating blood

B. Elevated 14-3-3 protein in CSF

C. Periodic sharp-wave complexes in EEG

D. Abnormal auditory evoked potentials

E. Hyperintensity of basal ganglia in T2-weighted MRI

4. Which of the following histologic changes characterize Creutzfeldt-Jakob disease?

A. Spongiform degeneration of the cerebral cortex

B. Neuronal losses

C. Eosinophilic cytoplasmic inclusions in the remaining neurons

D. Astrocytic proliferation

E. Amyloid/prion plaques in the cerebral cortex

STIONS -

5. Which of the following statements characterize fatal familial insomnia?

A. It is an autosomal recessive disorder.

B. The pathology is confined to the basal ganglia.

C. The pathology is confined to the thalamus.

D. The disease is associated with point mutation of PRNP at codon 178.

E. Autonomic dysfunctions are common.

6. The new variant of Creutzfeldt-Jakob disease is characterized by:

A. Behavioral changes and psychiatric symptoms usually introduce the disease

B. Cortical blindness is common in affected patients

C. T2-weighted MRI may show hyperintensity of pulvinars

D. Amyloid/prion plaques are abundant in the cerebral cortex

E. Perivascular lymphocytic infiltrations are prominent

(Answers are provided in the Appendix.)

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